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    Pantothenate Kinase Deficiency Related Neurodegeneration (PKAN) – An Ultra Rare Inherited Metabolic Disorder

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    Pantothenate Kinase-related Neurodegeneration (PKAN) is an Autosomal Recessive (AR) inherited disease identified by focal iron accumulation in the basal ganglia. Formerly recognized as Hallervorden-Spatz disease. PKAN is now considered to be one of several diseases that clinically presents with Neurodegeneration due to deposition of iron in brain (NBIA). Here, we describe an eleven-year-old boy identified as typical PKAN

    Clinical Profile and Predictors of Outcome of Guillain Barre Syndrome Variants among Children Admitted in the Pediatric Department of a Tertiary Care Hospital, Karachi

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    Background: Guillain Barre Syndrome (GBS) is one of the leading cause of Acute Flaccid Paralysis presenting in pediatric emergency, marking incidents in Asian countries as 3-4 per 100, 000 population. To improve outcome, an accurate and early recognition of predictors of bad prognosis is required. Objective: To identify requirement of intensive monitoring and aggressive treatment to help the health system in decreasing morbidity and mortality associated with Guillain Barre Syndrome. Materials and Methods: This is a cross-sectional study, conducted at Dr. RKMP Civil Hospital Karachi. Medical records of patients >3 months to 12 years of age, admitted with diagnosis of Guillian Barre syndrome from Jan 2017 to June 2022 were reviewed. Data was analyzed in SPSS version 23. For independent variables, frequency and percentage was used. Results: A total of 46 patients were enrolled in the study, mean age of study participants was 80.26 ± 31.7 months. The most common variant of GBS identified was AIDP in 19 (41.3%) followed by ASMAN 14 (30.4%). A positive association between winter season with 2.7 (95% CI 1.4-5.8) and progression within 7 days with 3.2 (95% CI 1.9-6.7) was found with mortality. Conclusion: Acute inflammatory demyelinating polyneuropathy was the most common variant of Gullian-Barre syndrome with male predominance. Presentation in winter seasons and progression of disease in less than 7 days are risk factors for mortality. Bulbar palsy is an independent risk factor for mechanical ventilation, irrespective of GBS variant

    Social and Cultural Norms; Still a Resistance to Nursing Care Homes for Older Patients in KPK, Pakistan

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    The percentage of the elderly population is increasing worldwide. The same trend is also observed in Pakistan. The elderly face many health problems in their advanced age. Elderly those who have chronic conditions like stroke need special and long-term care. So, for the provision of long-term care nursing care homes are crucial in a community. But, unfortunately in the province of Khyber Pakhtunkhwa, there is strong resistance to nursing care homes due to cultural and social norms

    Clinical Relationship of Blood Vitamin-C Levels and Age Related Cataract

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    Abstract: Background: Cataract is an extremely important and ground source of the curable blindness around the globe. Lenticular Vitamin C is actual important ultraviolet filter and antioxidant that decreases entry of the light rays into lens and therefore preventing the lens from oxidative damage. Keeping this in sight, higher intake of the vitamin c and by consuming the diet rich in vitamin c can prevent the age-related cataract. The motive of this study is to analyze if high vitamin C levels can lead to decrease in the percentage of cataract in patients. Objective: To determine occurrence of the senile cataract among Vitamin C deficient subjects and compare mean vitamin C levels among senile cataract and controls. Materials and Methods:  This comparative cross-sectional study was conducted at The Department of Ophthalmology, PIMS (Pakistan Institute of Medical Sciences), Islamabad, Pakistan, after being accepted by Ethical review Committee. Individuals were selected, in the age range between 45 to 75 years of age from the patients who visited Eye OPD, PIMS, and Islamabad. All participants undergone detailed ophthalmic examination that included visual acuity assessment through the Snellen’s chart, anterior segment examination on the slit lamp. Blood vitamin C level was assessed in both cases and controls. Information was recorded in the designated Proforma and data was analyzed using SPSS 23.0 version. Results: We enrolled 100 patients 50 in each group. Average age of our individuals in our case set was 60.96±9.57 years and in control set remained 59.66±8.92 years. In study group 25(50.0%) remained male and 25(50.0%) remained female. similarly, in control group 19(38.0%) were male and 31(62.0%) were female. The mean value of level of serum vitamin C in study group was 0.55±0.06 mg/dl and average value of level of the serum vitamin C in control group was 0.87±0.07 mg/dl. There was significant difference as p value is less than level of implication (p < 0.05). In cataractous individuals the level of the serum vitamin C was low as compared to control set. Conclusion: In this study, researchers determined that blood vitamin C may be a preventive component towards cataract development that is likely influenced by vitamin C consumption

    Gender-Based Violence and its Effect on Mother, Fetus and Newborn Baby: A Systematic Review

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    Abstract: Gender-based violence (GBV) is a major public health concern resulting in physical, psychological, sexual, and social issues. GBV during pregnancy may results in complicated and grave consequences for mother and unborn child. The prevalamce of GBV was reportedly higher in developing countries, indicating higher maternal and child mortalities. Effect of GBV on pregnancy has been reported to enhance the chances of prolonged labor, obstructed labor and in severe injuries higher incidents of miscarriages and abortions are also been reported. Moreover, fetal respiratory distress, diarrhea, small for gestaional age and Intra Uterine Growth Retardation (IUGR) have also been reported. This review aims to present a systematic view of genderbased violence and its association with adverse pregnancy outcomes and to contribute by filling the existing information gap. Review assessed the effect of GBV during pregnancy on fetal health, reporting infant diarrhea more frequently, along with fever, vomiting, and respiratory distress. The significant association between adverse maternal and fetal outcomes and gender-based violence has been evaluated extensively

    The Role of Hemogram Parameters in Predicting Sentinel Lymph Node Metastasis in Breast Cancer

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    Abstract: Background: Breast cancer remains the second most common cancer in the world after lung cancer. Breast cancer rate in Turkey was 40-50 / 100.000 in the west, and was 20 / 100.000 in the east. Objective: In our study, we aimed to investigate the prediction of hemogram parameters in showing sentinel lymph node metastasis. Materials & Methods: Patients undergoing sentinel lymph node biopsy (SLNB) with the diagnosis of breast cancer between January 2015 and August 2019 in General Surgery Department of Abant Izzet Baysal University were retrospectively analyzed in present descriptive study. The patients were divided into two groups, SLNB positive and negative. The measured hemogram parameters were compared between these two groups. Analysis of the data was held with a statistical software (SPSS 18.0 for Windows, IBM Inc., Chicago, IL, USA). Results: A total of 93 patients were included in the study. The average age of SLNB positive and negative patients were 55 (30-81) years 59 (35-88) years respectively. In detecting SLNB positivity, sensitivities and specificities of Tumor diameter> 0.9 cm were 72% sensitivity, 56% specificity, of CRP> 0.65 mg/dL were 76% sensitivity, 51% specificity, of RMR> 1.85 were 72% sensitivity, 46% specificity, of RDW> 15.45% were 75% sensitivity,% 42 specificity, of PLR> 106.5 were 72% sensitivity, 56% specificity. Conclusion: Since auxiliary metastasis is one of the most important points for the prognosis of breast cancer, we suggest PLR, RDW, RMR could serve as prognostic factors in determining lymph node metastasis along with CRP and tumor size in breast cancer

    Multi-Disciplinary Management of Hidradenocarcinoma

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    Hidradenocarcinoma makes up 6% of eccrine malignancies, and is an uncommon eccrine sweat gland tumour. The etiology is due to genetic alterations and transformation from an existing lesion or hidradenoma. It typically affects the face and scalp, although it can also cause nodal involvement and show up on the trunk, abdomen, or groin. Due to its similarities to other skin tumours, it is challenging to diagnose histopathologically. Therefore, immunohistochemical markers, such as p53, Ki-67, keratin AE1/AE3, and cytokeratin 5/6, are crucial for the diagnoses. There are no prospective randomized trials available to direct treatment of hidradenocarcinoma. A key component of therapy is early diagnoses and case discussion in multidisciplinary tumor board. Wide local excision with clear margins is the mainstay of treatment. Adjuvant treatment like chemo radiation therapy is incorporated if nodal disease or positive margins are present. Recurrences are treated by re-excision followed by adjuvant radiation therapy. Adjuvant hormonal therapy depends on the receptor status of the disease. These tumors have a 50% recurrence rate and up to 60% of the time they metastasize to the brain, bone, lung, liver, mediastinum, or peritoneum in a two-year period. The overall five year survival rate is around 30%. Keeping in view the aggressive course of this disease, research based clinically evident new treatment strategies are the need of time to optimize overall survival and local disease control

    The Faces of Deceptive Healers: A Scoping Review of Titles and Traits Associated with Quacks across Regions

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    Abstract: Background: The progress in medical science and the increased accessibility of digital resources have enhanced the convenience for individuals in accessing appropriate treatments. Despite these advancements, quackery continues to persist as a global concern. Throughout history, people have been intrigued by miraculous treatments rooted in faith and trust theories. In ancient times, distinguishing between scientifically approved and unapproved medical practices posed challenges. Ever since medical practices have been taking place, service providers took advantage of people’s unawareness, exploiting patients for personal gain. Over time, in certain cultures and societies, their intentions became apparent, and they earned a reputation as deceivers in the medical field. The current research seeks to uncover the various traits utilized by quacks across different times and locations worldwide, aiming to expose their fraudulent practices. Methodology: In this scoping review, the focus was on investigating various characteristics displayed by quacks during their practices. The study involved a comprehensive examination of 3740 published research articles and 400 pieces of gray literature such as websites of regulatory authorities, Acts, Laws. News items and reports which encompassed regulatory authority websites, news articles, and reports. From this extensive pool of sources, 45 articles and 4 excerpts were carefully chosen for in-depth analysis. Result: The current study highlighted 90 terms used for quacks to define their characteristics. The data was analyzed and developed five different themes defining various traits according to the near association. The main theme covered the moral values of these practitioners followed by Modern quackery under complementary and alternative healthcare services, sorcery and mystic, ignorant of science, and contract to law. Conclusion: Quackery, with its long-standing history, remains an ongoing health threat. Embracing modern technology, it utilizes the Internet and social media to propagate deceptive claims. Gaining insights into the traits of quacks is crucial for policymakers to address this issue effectively. Combating quackery necessitates heightened awareness, robust regulations, dissemination of authentic information and evidence-based healthcare involving all stakeholders

    Delayed Presentation of Traumatic anterior Sternoclavicular Joint Dislocation - A Case Report and Literature Review

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    Abstract: Traumatic sternoclavicular joint dislocations are uncommon, accounting for just 1% of all documented upper limb dislocations, despite the fact that upper limb dislocations are very common literature offers only a handful of documented instances of such dislocations. Among them, anterior dislocations are more frequently observed than posterior dislocations, owing to the robust ligamentous support in this area. We describe the case of a 35-year-old male patient who had isolated anterior sternoclavicular dislocation and a successful reduction procedure used on him. The rarity of such cases is emphasized by the satisfactory outcomes presented in our report

    Impact of Outpatient Quality Services on Patient Satisfaction

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    Abstract: Background: The success and survival of any healthcare organization depends on the patient’s satisfaction and quality. Patient satisfaction plays a vital role in the performance of care. Objective: To identify the factors influencing patient satisfaction with outpatient quality services in public and private hospitals. Materials and Methods: The study was conducted on outpatient patients from different public and private hospital settings in Karachi, Pakistan. Data was collected through virtual tools such as Google Docs, asking about their experience of outdoor patients (OPD) in public or private hospitals. Using a non-probability, convenience sampling technique, a 5% margin of error and a confidence interval of 95%, the sample size was calculated as 377 participants rounded off to 400. Respondents were divided into males and females who visited any specialty department or general OPD. A self-administered questionnaire was designed to gather data, and a digital tool such as Google Docs was used to reach a wider audience. The reliability of the pre-validated tools has been assessed through Cronbach’s alpha. The Cronbach alpha for outpatient quality services is 0.717, and patients’ satisfaction is 0.98.  The data were analyzed using the SPSS 25V by IBM Corporation. Result: Most respondents were from the age group (26-35 years), making up 42% of the entire population. Data gathered showed private hospitals preference over public hospitals and satisfaction as well. All the quality service variables identified in the study had a direct relationship with patient satisfaction except waiting time, which had a negative relationship. Regression Analysis showed a positive impact on the availability of services; thus 1% increase in service will lead to a 0.484% change in patient satisfaction. A negative relationship was also witnessed with a 1% increase in waiting time patient satisfaction is down by 0.119%. Conclusion: The level of satisfaction in public hospitals was not quite adequate, and the services were correspondingly rated as exceptional. The findings suggest that focusing on the nature of physician-related services, waiting for test results, and waiting for appointments are the most important factors impacting patient satisfaction in the outpatient division

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